Hepatoblastoma: Comprehensive Review With Recent Updates.

Abstract

Hepatoblastoma (HB), the most common primary malignant liver tumor of childhood, demonstrates remarkable histologic heterogeneity and can be classified into epithelial or mixed epithelial-mesenchymal subtypes. This review summarizes updates in histologic classification, molecular signatures, staging, and risk stratification of HB. The Children's Hepatic tumors International Collaboration represents an international effort to standardize the study of rare pediatric liver tumors; emphasis continues to remain on improving risk stratification by a combination of clinical, histologic, and molecular features to tailor treatment in a bid to reduce toxicity while maintaining or improving efficacy. Pure fetal HB is cured by complete resection without the need for adjuvant chemotherapy. Malignant rhabdoid tumors have been parsed out from small cell undifferentiated HBs by negative INI-1 staining on immunohistochemistry; these tumors require a distinct and more aggressive chemotherapeutic regimen. The significance of recently characterized "blastema" component in HB remains to be elucidated. Hepatocellular neoplasm, not otherwise specified, is a provisional diagnostic category for tumors exhibiting either intermediate or a combination of both HB and hepatocellular carcinoma histologic features. The Children's Hepatic tumors International Collaboration risk stratification algorithm includes age as an important discriminator of risk, in addition to AFP, metastasis, and PreTreatment EXTent of disease stage and its annotations.